CH6 - 3) Chronic Leukemia Flashcards
What is chronic leukemia?
Neoplastic proliferation of mature circulating lymphocytes
What is chronic leukemia characterized by?
a high WBC count
Describe the onset of chronic leukemia?
Usually insidious in onset and seen in older adults
What is chronic lymphocytic leukemia?
Neoplastic proliferation of naive B cells that co-express CD5 and CD20;
What is the most common leukemia overall?
Chronic lymphocytic leukemia (CLL)
In CLL what is seen on the blood smear?
Increased lymphocytes and smudge cells are seen on blood smear
small lymphocytic lymphoma
In CLL, with involvement of lymph nodes leads to generalized lymphadenopathy
What are the complications of CLL?
1) Hypogammaglobulinemia, 2) autoimmune hemolytic anemia, 3) Richter transformation
What is the most common of death in CLL?
infection
What is Richter transformation?
transformation to diffuse large B-cell lymphoma
What is Richter transformation marked clinically by?
an enlarging lymph node or spleen
What is Hairy cell leukemia?
Neoplastic proliferation of mature B cells
What characterizes hairy cell leukemia?
hairy cytoplasmic processes
What are hairy cell leukemia cells positive for?
tartrate-resistant acid phosphatase (TRAP).
What are the clinical features for hairy cell leukemia?
Splenomegaly, dry tap on bone marrow aspiration
Why is there splenomegaly in hairy cell leukemia?
Due to accumulation of hairy cells in red pulp
Why is there a dry tap on bone marrow aspiration in hairy cell leukemia?
due to marrow fibrosis
How is lymphadenopathy related to hair cell leukemia?
Lymphadenopathy is usually absent
What drug is used in the treatment of hairy cell leukemia?
2-CDA (cladribine)
How does hair cell leukemia respond to cladribine?
excellent response
How does cladribine work?
Its an adenosine deaminase inhibitor, adenosine accumulates to toxic levels in neoplastic B cells.
What is adult T cell leukemia?
Neoplastic proliferation of mature CD4+ T cells (ATLL)
What is adult T cell leukemia associated with?
Associated with HTLV-I (human T cell lymphotrophic virus -1)
Where is ATLL associated with HTLV-1 most commonly seen in?
Japan and the Caribbean
What are the clinical features for ATLL?
include rash (skin infiltration), generalized lymphadenopathy with hepatosplenomegaly, and lytic (punched-out) bone lesions with hypercalcemia
What is mycosis fungoides?
Neoplastic proliferation of mature CD44 T cells that infiltrate the skin, producing localized skin rash, plaques, and nodules
What are Patitrier microabscesses?
Aggregates of neoplastic cells in the epidermis
Sezary syndrome
cells can spread to involve the blood, producing Sezary syndrome.
What are Sezary cells?
Characteristic lymphocytes with cerebri form nuclei
What would you see on blood smear in mycosis fungoides?
Sezary cells